He aa koko ko kāu pēpē i loko o kona puʻuwai e like me ka pahi kaua? E aʻo kākou e pili ana i kēia maʻi laha ʻole (Scimitar Syndrome)!

"ʻAuē, me he mea lā ke pilikia nei kā mākou keiki liʻiliʻi i ka hanu ʻana, ke hanu nei ʻo ia ʻoiai ʻo ia e hānai waiū ana... i kekahi manawa ua uliuli kona ʻili..." Ua ʻike anei ʻoe i kekahi mea e like me kēia? A i ʻole ua ʻike anei ʻoe i kēia e hana ana i ka pēpē a kekahi āu i ʻike ai? I kekahi manawa hiki ke hana ʻia kēia mau hōʻailona e kekahi mau pilikia puʻuwai i hānau ʻia me nā pēpē. I kēia lā e kamaʻilio mākou e pili ana i kekahi ʻano maʻi puʻuwai kakaʻikahi, akā he mea nui e pono ke ʻike.

He aha ka maʻi Scimitar?

ʻO kēia ka maʻi Scimitar . He ʻano ʻē iki ke kani ʻana o ka inoa, ʻeā? ʻO 'Scimitar' e pili ana i ka pahi kaua me ka wēlau piʻo, i hoʻohana ʻia i ka wā kahiko ma ka Hikina Waena. I kēia kūlana, i kekahi manawa ʻike ʻia ka aʻa pulmonary, ka mea e lawe ana i ke koko mai ka māmā ʻākau a i ka puʻuwai, ma ke ʻano o ka pahi kaua piʻo ma nā kukuna X. ʻAʻole anei he mea hoihoi loa ke ʻano o ka inoa i loaʻa mai ai?

I ka ʻōlelo maʻalahi, he kīnā puʻuwai hānau kēia. ʻO ia hoʻi, he kūlana e kū mai ana i ka wā e noho ana ke pēpē i loko o ka ʻōpū. Ua kapa ʻia kēia e nā kauka he ʻano kūlana i kapa ʻia ʻo Partial Anomalous Pulmonary Venous Return (PAPVR). ʻO ia hoʻi, aia kahi kīnā i ke ala e pili ai nā kīʻaha koko mai nā māmā i ka puʻuwai.

Aia kekahi mau hōʻailona nui e ʻike ʻia ma kēia kūlana:

• ʻAʻole i ulu piha ka māmā ʻākau (hypoplastic right mum). ʻO ke ʻano kēia, ua liʻiliʻi a ʻaʻole hana pono ma mua o ka māmā ma kēlā ʻaoʻao.
• ʻAʻole i hoʻomohala pono ʻia ke aʻa nui e lawe ana i ke koko i ka māmā ʻākau (ʻaʻa pulmonary ʻākau) (ʻaʻa pulmonary hypoplastic ʻākau). Hiki i kēia ke hōʻemi i ka nui o ke koko e hiki ana i ka māmā ʻākau.

Hiki iā kākou ke hoʻomaopopo iki e pili ana i nā hana kupaianaha o ko kākou mau naʻau?

No laila, i mea e maopopo ai i ka mea e hana nei me ka Scimitar Syndrome, e nānā mua kākou pehea e hana ai ka puʻuwai olakino, ʻae? E noʻonoʻo i ko kākou puʻuwai he pamu hana maikaʻi loa. Hoʻopuka ia i ke koko ma ko kākou kino holoʻokoʻa, i ke ao a me ka pō.

ʻO kēia ka mea maʻamau e hana ʻia:

1. Hoʻohana ʻia, hōʻiliʻili ʻia ke koko nele i ka oxygen mai ke kino holoʻokoʻa a hoʻihoʻi ʻia i ka ʻaoʻao ʻākau o ka puʻuwai.

2. Hoʻouna ʻia kēia koko nele i ka oxygen mai ka ʻaoʻao ʻākau o ka puʻuwai i nā māmā, ma o nā aʻa pulmonary .

3. I loko o nā māmā, ua 'ʻokikene ʻia' kēia koko, e like me ka wai lepo i kānana ʻia a hoʻomaʻemaʻe ʻia.

4. A laila, hoʻi kēia koko waiwai i ka oxygen i ka ʻaoʻao hema o ka puʻuwai ma o nā aʻa pulmonary, ʻo ia hoʻi nā aʻa e hele mai ana mai nā māmā a i ka puʻuwai .

5. ʻO ka ʻaoʻao hema o ka puʻuwai e pamu i kēia koko waiwai i ka oxygen i ke kino holoʻokoʻa, ma o ke kīʻaha koko nui loa, ʻo ka aorta .

Penei ke ala e loaʻa ai ka oxygen i kēlā me kēia ʻāpana o ko kākou kino. ʻAʻole anei kēia he hana paʻakikī a kupaianaha hoʻi?

No laila he aha ka mea e hana ai ma ka Scimitar Syndrome?

I kēia manawa, ua maopopo paha iā ʻoe pehea e hana ai ka puʻuwai olakino. ʻO ka mea e hana ʻia i loko o ke kanaka me Scimitar Syndrome, ʻo ke koko e pono e hele mai ana mai nā māmā e hōʻiliʻili i ka oxygen a hele i ka ʻaoʻao hema o ka puʻuwai, ma kahi o ka hele ʻana mai i kēlā ʻaoʻao , hele hewa ia a hele i ka ʻaoʻao ʻākau o ka puʻuwai.

E noʻonoʻo inā ʻoe e hoʻopili hou i kahi paipu e lawe mai ana i ka wai i kahi hale i kahi kūpono ʻole, malia paha i kahi pahu wai i hoʻohana ʻia. A laila ʻaʻole e hana pono ʻia ka mea e pono ai ke hana. Hiki ke kū mai kahi kūlana kahi e hui pū ʻia ai ka wai maʻemaʻe a me ka wai lepo.

Ma kēia, hoʻokahi a ʻoi aku paha nā aa koko (nā aa koko pulmonary) e lawe maʻamau ana i ke koko oxygenated mai ka māmā ʻākau e pili ana i ka ʻaoʻao ʻākau o ka puʻuwai ma kahi o ka ʻaoʻao hema. ʻAe kēia i kekahi o ke koko oxygenated e kahe hou i nā māmā. Hoʻoikaika pū ia i ka hana ʻana o ka ʻaoʻao ʻākau o ka puʻuwai.

I kekahi manawa, ʻaʻole loaʻa ke koko i ka māmā ʻākau mai nā aʻa pulmonary , akā pololei mai ka aorta . ʻO ka aorta ke kīʻaha koko nui e lawe ana i ke koko i ke kino holoʻokoʻa. No laila, aia kekahi pilikia liʻiliʻi ma laila. Hiki i kēia ke kau i ke kaumaha pono ʻole ma ka puʻuwai.

Pehea ka maʻamau o kēia kūlana? Pono anei kākou e makaʻu pono ʻole?

He mea weliweli paha kēia iā ʻoe, akā he maʻi laha ʻole ka Scimitar Syndrome. Wahi a nā helu helu, hoʻokahi a ʻekolu wale nō pēpē e pili ana i kēlā me kēia 100,000. Ua ʻike ʻia hoʻi he pālua ka nui o ia mea i nā kaikamahine ma mua o nā keikikāne.

No laila, mai hopohopo nui, no ka mea, he mea kakaikahi loa kēia. Eia nō naʻe, he mea nui loa ka makaʻala i kēia.

He aha nā kumu no kēia? No ke aha lā kēia e hana nei?

I kekahi manawa, paʻakikī i nā kauka ke kuhikuhi i ke kumu pololei o ka Scimitar Syndrome. Eia nō naʻe, aia kekahi mau mea pilikia e hiki ke hoʻonui i ka pilikia o kahi pēpē i ka loaʻa ʻana o kahi kīnā puʻuwai hānau. ʻO kēia mau mea:

• Genetics: I kekahi manawa hiki i nā kūlana genetic i hoʻoili ʻia ma o nā hanauna ke loaʻa ka hopena.
• ʻO kekahi mau lāʻau lapaʻau i lawe ʻia i ka wā hāpai: No ka laʻana, hiki i kekahi mau lāʻau lapaʻau ʻākena e like me `(Isotretinoin - Accutane®)` ke lilo i mea weliweli. ʻO ia ke kumu i ʻōlelo ʻia ai ʻaʻole e lawe i kekahi lāʻau lapaʻau i ka wā hāpai me ka ʻole o ke aʻo ʻana a ke kauka.
• Nā kumu o ke kaiapuni: Inā puhipaka ka makuahine a inu waiʻona paha i ka wā hāpai, hiki i kēlā mau mea ke hoʻopilikia i ka ulu ʻana o ka puʻuwai o ka pēpē.
• ʻO kekahi mau maʻi i ka makuahine: No ka laʻana, inā loaʻa i ka makuahine kahi maʻi e like me ka rubella i ka wā hāpai, hiki iā ia ke hoʻopilikia i ka ulu ʻana o ka puʻuwai o ka pēpē.
• Nā maʻi maʻi mau loa o ka makuahine i kāohi ʻole ʻia: Inā ʻaʻole i kāohi pono ʻia nā maʻi e like me ka maʻi diabetes mellitus i ka wā hāpai, hiki iā ia ke hoʻopilikia i ka pēpē.

ʻO ka mea nui, i ka hapa nui o ka manawa, ʻaʻole kēia he hewa o kekahi. No laila, mai hoʻāhewa iā ʻoe iho.

He aha nā hōʻailona e kū nei?

ʻAʻole like nā hōʻailona o ka Scimitar Syndrome no kēlā me kēia kanaka. Hiki i kekahi mau pēpē ke hōʻike i nā hōʻailona ma hope koke o ka hānau ʻana . ʻO nā mea e makaʻala ai i kēlā mau manawa:

• Ka paʻakikī o ka hanu ʻana a me ka ʻū ʻana i ka wā e hānai ai i ka waiū.
• ʻO ke kala ʻana o ka ʻili a me nā lehelehe i polū/hina (cyanosis): ʻO ke kumu o kēia, ʻaʻole lawa ka oxygen i ke kino.
• Ka hanu wikiwiki ʻana.
• Pehu (edema) ma nā wāwae, ka ʻōpū, a puni nā maka.

Eia nō naʻe, ʻaʻole paha e hōʻike kekahi mau pēpē i kekahi mau hōʻailona a hiki i ko lākou wā ʻelemakule. Hiki i kekahi ke noho asymptomatic a hiki i ka wā makua. Eia nō naʻe, inā ʻike ʻia nā hōʻailona i ka wā makua, loaʻa paha iā lākou:

• Nā maʻi pinepine o ka māmā.
• Ka paʻakikī o ka hanu ʻana (dyspnea) i ka wā e hoʻoikaika kino ai a i ʻole ka hoʻoikaika kino.

Pehea e ʻike ai nā kauka i kēia? (Diagnosis)

He nui nā hoʻokolohua a nā kauka e kōkua ai i ka ʻike ʻana i ka Scimitar Syndrome. Hiki i kēia ke nānā i nā mea e like me ka nui o ka māmā ʻākau i loko o kahi pēpē a makua paha a me ke kūlana o ke aʻa pulmonary ʻākau.

• X-ray umauma: Hiki i kēia ke hāʻawi i kahi manaʻo maʻamau o ka puʻuwai, nā māmā, nā ala ea, a me nā kīʻaha koko. E ʻike paha ʻoe i ke ʻano 'Scimitar' ma kēia.
• CT scan: Lawe kēia i nā kukuna X mai nā kihi like ʻole e hana i nā kiʻi kikoʻī ʻekolu-dimensional o loko o ke kino.
• MR angiography: He hoʻāʻo kēia e hoʻohana ana i nā nalu magnetic a me kahi kamepiula e nānā pono i nā kīʻaha koko.
• ʻO ka echocardiogram Transthoracic (TTE): Ua like kēia me kahi scan ultrasound. Hiki iā ia ke nānā pono i ka hana o ka puʻuwai, nā keʻena, nā valves, a me ke kahe koko.
• Catheterization cardiac: Ma kēia kaʻina hana, hoʻokomo ʻia kahi dye kūikawā i loko o nā kīʻaha koko a lawe ʻia kahi X-ray e ʻike ai pehea e kahe ai ke koko ma o nā kīʻaha koko. Hoʻohana ʻia nō hoʻi ia e ana i ke kaomi i loko o ka puʻuwai a me nā māmā.

Mai makaʻu e nīnau e pili ana i kēia mau hoʻokolohua. Hana nā kauka i kēia mau mea e ʻike pololei ai i ke kūlana a hāʻawi i ka lāʻau lapaʻau maikaʻi loa.

Aia kekahi lāʻau lapaʻau no kēia? (Nā ʻano lapaʻau)

ʻAe, ʻo ke ʻoki kino ke ʻano lapaʻau nui no ka Scimitar Syndrome.

• No nā pēpē, inā ʻike nā kauka i kēia kūlana, hoʻowalewale pinepine ʻia lākou e hana i ke ʻoki kino i loko o nā mahina ʻelua mua o ke ola . No ka mea, ʻo ka wikiwiki o ka hoʻoponopono ʻana i kēia kīnā, ʻoi aku ka maikaʻi no ka ulu ʻana o ka pēpē a me ke olakino i ka wā e hiki mai ana.
• No nā mākua,ʻAʻole pono nā kānaka a pau me ka Scimitar Syndrome i ke ʻoki kino ke ʻole lākou e loaʻa nā hōʻailona koʻikoʻi (e.g., nā maʻi pinepine o ka māmā, ka pōkole o ka hanu). Eia nō naʻe, inā koʻikoʻi nā hōʻailona, ​​​​pono paha ke ʻoki kino.

Aia kekahi mau mea e hiki i ke kauka lapaʻau ke hana i ka wā o ke ʻoki ʻana:

• ʻO ke aʻa pulmonary, ka mea i hoʻopili ʻia ma kahi kūpono ʻole , ua hoʻihoʻi ʻia i ka ʻaoʻao hema o ka puʻuwai. I kekahi manawa hoʻohana ʻia nā ʻāpana kūikawā no kēia.
• A i ʻole, ua hoʻokomo pololei ʻia ke aʻa koko i ka ʻaoʻao hema o ka puʻuwai.
• He mea kākaʻikahi loa, hiki ke hana ʻia kahi pneumonectomy e wehe loa i ka māmā ʻākau i ulu pono ʻole a hana pono ʻole. Eia nō naʻe, hana ʻia kēia ke ʻole he koho ʻē aʻe.

Aia kekahi ala e hiki ai iā kākou ke pale aku i kēia?

ʻO ka ʻoiaʻiʻo, ʻaʻohe ala e pale loa ai i kahi kīnā puʻuwai hānau e like me Scimitar Syndrome, ʻoiai ua hoʻopili ʻia hoʻi e nā kumu genetic.

Eia nō naʻe, aia nā mea hiki i ka makuahine ke hana i ka wā hāpai e hoʻonui i kona manawa e loaʻa ai kahi pēpē olakino:

• E pale loa i ka waiʻona, nā cigarette, a me nā lāʻau lapaʻau ʻē aʻe i ka wā hāpai.
• Ma mua o ka lawe ʻana i kekahi lāʻau lapaʻau, e kamaʻilio me kāu kauka e pili ana i kona mau pilikia a me nā pono.
• Inā ʻaʻole ʻoe i loaʻa i ka lāʻau pale rubella , he mea nui e loaʻa iā ia ma mua o kou hāpai ʻana, no ka mea, ʻo ka loaʻa ʻana o ka rubella i ka wā hāpai hiki ke hoʻopilikia i ka puʻuwai o ka pēpē.
• Inā loaʻa iā ʻoe nā maʻi mau e like me ka maʻi diabetes , e kaohi pono iā lākou i ka wā hāpai.
• E lawe i ka waikawa folic a me nā huaora prenatal ʻē aʻe e like me ke kauoha a kāu kauka.

Pehea lā ke ʻano o ke ola me kēia kūlana? (Hiʻohiʻona no ka wā e hiki mai ana)

ʻO ka poʻe e hana ʻia ana i ke ʻoki kino no ka Scimitar Syndrome he mau hopena maikaʻi loa ko lākou no ka wā lōʻihi . ʻAʻole ʻike hou ka hapa nui o ka poʻe i kekahi mau hōʻailona ma hope o ke ʻoki kino.

Eia nō naʻe, i kekahi manawa, ʻoi aku hoʻi i nā pēpē a me nā kamaliʻi ʻōpio, hiki i ka aʻa pulmonary i hoʻololi ʻia ke ala e lilo i mea haiki. Inā hana ʻia kēia, pono paha ka lāʻau hou aʻe.

Hiki i kekahi mau pākeke me ka Scimitar Syndrome ke ola olakino me ka ʻole o nā hōʻailona. Eia nō naʻe, no nā pākeke ʻē aʻe, hiki i ke kūlana ke alakaʻi i nā pilikia puʻuwai ʻē aʻe. I kēlā mau hihia, hiki ke hoʻopilikia ʻia ke ʻano o ke ola. Inā loaʻa nā hōʻailona (e like me ka pōkole nui o ka hanu, nā maʻi māmā), pono paha ke ʻoki ʻana. Pono paha ʻoe e loaʻa nā hoʻokolohua kūikawā e nānā i ke koko kiʻekiʻe i loko o nā aʻa i loko o kou mau māmā.

Aia kekahi mau maʻi puʻuwai ʻē aʻe e pili ana me ka Scimitar Syndrome?

ʻAe, he nui ka poʻe me Scimitar Syndrome e loaʻa paha i nā pilikia cardiovascular ʻē aʻe. Hānau ʻia kekahi me nā kīnā puʻuwai he nui. A i ʻole, hiki i ka Scimitar Syndrome ke hoʻoulu i nā pilikia ʻē aʻe.

ʻO nā kūlana ʻē aʻe e pili ana paha:

• ʻO ke kīnā o ka septal atrial (ASD): He lua ma waena o nā keʻena ʻelua o luna o ka puʻuwai.
• Mama holo lio: He pilina maʻamau ʻole ma waena o nā ʻāpana ʻelua o ka mama.
• Ke koko kiʻekiʻe o ka puʻuwai: Ke kaomi kiʻekiʻe i loko o nā aʻa o nā māmā.
• Ka hoʻokaʻawale ʻana o ka pulmonary (māmā kōkua): He ʻāpana hana ʻole, hoʻokaʻawale ʻia o ka ʻiʻo māmā me kahi lako koko kūpono ʻole.
• ʻO Tetralogy o Fallot: He kīnā puʻuwai maʻamau e pono ai ka mālama ʻana e pamu i ke koko i nā māmā i nā pēpē.
• ʻO ke kīnā septal ventricular (VSD): He lua ma waena o nā keʻena pamu ʻelua o lalo o ka puʻuwai.

Hiki i ka lohe ʻana i kēia mau mea ke lilo i mea weliweli hou aku. Eia nō naʻe, aia nā lāʻau lapaʻau no kēia mau kūlana āpau. ʻO ka mea nui loa, ʻo ke kamaʻilio ʻana me kāu kauka, e hoʻomaopopo i kou kūlana pololei, a e loaʻa i ka lāʻau āu e pono ai.

Nā nīnau koʻikoʻi e nīnau aku i kāu kauka

Inā ʻike ʻoe ua loaʻa iā ʻoe a i ʻole kāu keiki ka Scimitar Syndrome, hiki iā ʻoe ke nīnau i kāu kauka i nā nīnau penei:

• He aha ke kumu kūpono loa o ka Scimitar Syndrome?
• Pono anei au/kaʻu keiki i ke ʻoki kino?
• He aha ka mea e hana ʻia inā ʻaʻole ʻoe e hana ʻoki kino?
• Pehea e mālama ai i ke olakino o ka puʻuwai me ka Scimitar Syndrome?
• Inā loaʻa iaʻu kēia maʻi, he aha nā manawa kūpono e loaʻa pū ai i kaʻu keiki?
• Inā loaʻa iaʻu kaʻu keiki mua me ka Scimitar Syndrome, he aha nā manawa kūpono e loaʻa pū ai i kaʻu keiki ʻelua?

Mai makaʻu a hilahila paha e nīnau i kēia mau nīnau. He mea nui loa ia e hoʻomaʻemaʻe i nā kānalua a pau i loko o kou manaʻo.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

He kīnā puʻuwai laha ʻole ka Scimitar Syndrome i hānau ʻia e hoʻopilikia nui ana i ka māmā ʻākau a me nā kīʻaha koko e hoʻopili ai i nā māmā.

ʻAʻole paha e hōʻike kekahi mau pēpē i kekahi mau hōʻailona, ​​aʻo kekahi poʻe e pono ai ke ʻoki ʻana i loko o nā mahina ʻelua mua o ke ola.

ʻO nā pēpē e pono ai ke ʻoki ʻana i ka wā mua e pono paha i ka mālama hou ʻana ma hope no ka mea ua emi ke kīʻaha koko i hoʻohuli ʻia. Eia nō naʻe, he nui nā pākeke a me nā keiki i hana ʻia ke ʻoki ʻana no ka Scimitar Syndrome e ola kino maikaʻi loa a ola i nā ola maʻamau.

Lana ko mākou manaʻo he kōkua kēia ʻike iā ʻoe. Inā he mau nīnau hou aku kāu e pili ana i kēia, e kamaʻilio me kāu kauka. E noho olakino!

`ʻO ka maʻi Scimitar, maʻi puʻuwai, nā kīnā hānau, nā māmā, ke kahe koko, nā hōʻailona o ka pēpē, ke ʻoki kino